Restrictive allograft syndrome and idiopathic pleuroparenchymal fibroelastosis: do they really have the same histology?

AIMS: Restrictive allograft syndrome (RAS) and idiopathic pleuroparenchymal fibroelastosis (IPPFE) are two different diseases reported to share the same histology. RAS relates to chronic allograft dysfunction in lung transplantation, with IPPFE being a rare condition in native lungs. Our aim is to compare their histologies alongside biopsies of usual interstitial pneumonia (UIP), to determine if there are differences that might help to elucidate the pathogenesis. METHODS AND RESULTS: We selected four postmortem allograft lungs from patients who developed a clear clinical RAS pattern, five biopsies diagnosed as IPPFE, five UIP biopsies and five sections of normal lung. Histopathological features were described without knowledge of clinical and radiological features. Both RAS allografts and IPPFE biopsies showed intra-alveolar fibrosis and elastosis (IAFE), but RAS allografts also showed concomitant obliterative bronchiolitis, vascular lymphoplasmacytic inflammation within fibrointimal thickening, less fibroblastic foci (FF) at the advancing edge of the fibrosis (one against 14.4 FF in 2 mm2 ) and a slight reduction of the capillary network compared to UIP (P = 0.07) and controls (P = 0.06). The main differences seen in UIP were the lack of IAFE and the presence of honeycomb change. CONCLUSIONS: RAS and PPFE histopathology both show IAFE, but display various differences, particularly in their vascular morphology that may allow further understanding of pathogenesis.

Development of a PET/CT and PET/MRI patient information films in collaboration with patient representatives previously treated for cancer.

Introduction: PET/MRI and PET CT scanning carries an increased psychological burden for patients which may be improved by effective preparation prior to attendance for the procedure. We aimed to devise an online resource for patient benefit prior to PET/MRI and PET/CT scanning. Methods: With the help of patient representatives' the style and content for two patient information films was reviewed at each stage of the process to explain the patient journey. Results: Two patient information films were produced for patients undergoing PET scanning, Positive feedback from healthcare providers and users was obtained. Conclusion: Involvement of patient representatives in the production of an information resource has had a positive impact on the finished product, with potential for increased benefit to patients as a result.